Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue.

2.50
Hdl Handle:
http://hdl.handle.net/10541/94754
Title:
Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue.
Authors:
Banerjee, Saumitra S; Agbamu, D A; Eyden, Brian P; Harris, Martin
Abstract:
AIMS: To study the clinical and pathological features of primary malignant peripheral primitive neuroectodermal tumours (PNETs) of the skin and subcutaneous tissue, to discuss the differential diagnosis, and to review the existing literature on these tumours. METHODS AND RESULTS: Eight cases of PNETs presenting in the skin and subcutaneous tissue were identified from the pathology records of the Christie Hospital, Manchester. Detailed immunohistochemical studies were performed on all cases and seven tumours were subjected to electron microscopic examination. Detailed clinical and follow-up information was obtained on seven cases. Six tumours occurred in children and adolescents and two were seen in young adults (age range, 8-36 years). No sex or site predilection was observed. Five tumours occupied the dermis and subcutis and three were entirely located in the subcutaneous tissue. Microscopically, they were composed of small round cells and seven tumours contained glycogen. Only one tumour focally exhibited Home-Wright rosettes and neuropil. Two tumours contained rhabdoid or plasmacytoid cells in places and all cases showed microcystic and pseudovascular spaces. Immunostains revealed MIC2 (8/8), NSE (7/8), PGP9.5 (7/8), beta 2 microglobulin (7/8), neurofilament protein (6/8), S100 protein (3/8), synaptophysin (2/8) and Leu-7 (1/8) positivity. Anomalous cytokeratin (4/8), desmin (2/8), myoglobin (2/8), NKIC3 (4/8) and GFAP (1/8) staining was also noted. Ultrastructurally, neuroendocrine granules were detected in five cases and one case exhibited microtubules in processes. Adequate follow-up information was available in four cases. One patient died of metastatic disease. One child developed axillary lymph node metastasis but is alive with no evidence of disease 96 months after treatment. Two other patients are alive with no residual or recurrent disease 44 and 52 months after excision and radio/chemotherapy. CONCLUSION: PNETs are rare malignant small round cell tumours of the skin and subcutaneous tissue which are probably underdiagnosed. A correct diagnosis can be made on light microscopic features, demonstration of neuroendocrine granules on electron microscopy and a combination of MIC2, beta 2 microglobulin, and more than one neural marker positivity. These neoplasms should be differentiated from other cutaneous neoplasms composed of small round cells. The number of cases of cutaneous PNETs studied so far is rather small, and no firm conclusion can be drawn about their behaviour but long-term survival is possible in some cases.
Affiliation:
Department of Histopathology, Christie Hospital, Manchester, UK.
Citation:
Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue. 1997, 31 (4):355-66 Histopathology
Journal:
Histopathology
Issue Date:
Oct-1997
URI:
http://hdl.handle.net/10541/94754
DOI:
10.1046/j.1365-2559.1997.2770865.x
PubMed ID:
9363452
Type:
Article
Language:
en
ISSN:
0309-0167
Appears in Collections:
All Christie Publications

Full metadata record

DC FieldValue Language
dc.contributor.authorBanerjee, Saumitra Sen
dc.contributor.authorAgbamu, D Aen
dc.contributor.authorEyden, Brian Pen
dc.contributor.authorHarris, Martinen
dc.date.accessioned2010-03-23T16:45:09Z-
dc.date.available2010-03-23T16:45:09Z-
dc.date.issued1997-10-
dc.identifier.citationClinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue. 1997, 31 (4):355-66 Histopathologyen
dc.identifier.issn0309-0167-
dc.identifier.pmid9363452-
dc.identifier.doi10.1046/j.1365-2559.1997.2770865.x-
dc.identifier.urihttp://hdl.handle.net/10541/94754-
dc.description.abstractAIMS: To study the clinical and pathological features of primary malignant peripheral primitive neuroectodermal tumours (PNETs) of the skin and subcutaneous tissue, to discuss the differential diagnosis, and to review the existing literature on these tumours. METHODS AND RESULTS: Eight cases of PNETs presenting in the skin and subcutaneous tissue were identified from the pathology records of the Christie Hospital, Manchester. Detailed immunohistochemical studies were performed on all cases and seven tumours were subjected to electron microscopic examination. Detailed clinical and follow-up information was obtained on seven cases. Six tumours occurred in children and adolescents and two were seen in young adults (age range, 8-36 years). No sex or site predilection was observed. Five tumours occupied the dermis and subcutis and three were entirely located in the subcutaneous tissue. Microscopically, they were composed of small round cells and seven tumours contained glycogen. Only one tumour focally exhibited Home-Wright rosettes and neuropil. Two tumours contained rhabdoid or plasmacytoid cells in places and all cases showed microcystic and pseudovascular spaces. Immunostains revealed MIC2 (8/8), NSE (7/8), PGP9.5 (7/8), beta 2 microglobulin (7/8), neurofilament protein (6/8), S100 protein (3/8), synaptophysin (2/8) and Leu-7 (1/8) positivity. Anomalous cytokeratin (4/8), desmin (2/8), myoglobin (2/8), NKIC3 (4/8) and GFAP (1/8) staining was also noted. Ultrastructurally, neuroendocrine granules were detected in five cases and one case exhibited microtubules in processes. Adequate follow-up information was available in four cases. One patient died of metastatic disease. One child developed axillary lymph node metastasis but is alive with no evidence of disease 96 months after treatment. Two other patients are alive with no residual or recurrent disease 44 and 52 months after excision and radio/chemotherapy. CONCLUSION: PNETs are rare malignant small round cell tumours of the skin and subcutaneous tissue which are probably underdiagnosed. A correct diagnosis can be made on light microscopic features, demonstration of neuroendocrine granules on electron microscopy and a combination of MIC2, beta 2 microglobulin, and more than one neural marker positivity. These neoplasms should be differentiated from other cutaneous neoplasms composed of small round cells. The number of cases of cutaneous PNETs studied so far is rather small, and no firm conclusion can be drawn about their behaviour but long-term survival is possible in some cases.en
dc.language.isoenen
dc.subjectNeuroectodermal Tumoursen
dc.subjectSkin Canceren
dc.subjectBiological Tumour Markersen
dc.subject.meshAdolescent-
dc.subject.meshAdult-
dc.subject.meshAntigens, CD-
dc.subject.meshCell Adhesion Molecules-
dc.subject.meshChild-
dc.subject.meshCytoplasmic Granules-
dc.subject.meshDiagnosis, Differential-
dc.subject.meshFemale-
dc.subject.meshHumans-
dc.subject.meshImmunohistochemistry-
dc.subject.meshMale-
dc.subject.meshNeuroectodermal Tumors, Primitive, Peripheral-
dc.subject.meshSarcoma, Ewing's-
dc.subject.meshSkin Neoplasms-
dc.subject.meshTumor Markers, Biological-
dc.titleClinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue.en
dc.typeArticleen
dc.contributor.departmentDepartment of Histopathology, Christie Hospital, Manchester, UK.en
dc.identifier.journalHistopathologyen
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